Ординатура / Офтальмология / Учебные материалы / Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatment

13 Neuro-Ophthamology 381

A B

Fig . 13.10 (A) Coloboma of the optic disk. (B) Morning glory syndrome. (Courtesy of

Robin D. Hamilton, A.M. Hamilton)

Presentation

Visual acu it y m ay be variably affected . Oph th alm oscopy reveals an en larged, p in k disk sit u ated in a fun n el-sh aped excavat ion w ith overlying glial t issu e. Ret in al blood vessels are in creased in n u m ber, arise from th e disk periph er y, an d ru n an abn orm ally st raigh t cou rse w ith acu te bran ch ing. Th e m acu la m ay be in corporated in th e postglial excavat ion (m acular capt u re). Th ere is an in creased risk of ret in al detach m en t . Associat ion s in clu de t ran ssph en oidal en ceph alocele, ipsilateral in - t racran ial vascu lar hyp oplasia, hypopit u it arism , an d facial abn orm alit ies-hyper- telorism , depressed n asal bridge, an d m idlin e u pper lip n otch cleft palate.

Management

Man agem en t con sists of regular follow -up an d pat ien t assu ran ce.

Tilted Disk

Tilted disk is bilateral, n on h ereditar y, presum ably du e to ect asia of th e in feron asal fun du s.

Presentation

Pat ien ts p resen t w ith an obliqu ely placed disk w ith a posteriorly displaced in fero- n asal port ion an d sit us inversu s of th e vessels. Bilateral h em ian opia th at does n ot resp ect th e ver t ical m eridian is seen . Myopic ast igm at ism is p resen t . Th e con di- t ion is associated w ith visu al-field defects. It m ay be associated w ith congen it al su prasellar t u m or.

Management

Man agem en t con sists of correct ion of th e refract ive error an d pat ien t assu ran ce.

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Optic Disk Pathology

Optic Neuritis

In flam m at ion of th e opt ic n er ve m ay be a ret robu lbar n eu rit is w ith n orm al opt ic disk (m ost frequen tly m ult iple sclerosis, young adult s), papillit is w h ere th e disk is sw ollen (m ost com m on ly in ch ildren ), or n eu roret in it is-associated in flam m at ion of ret in al n er ve fibers w ith m acu lar st ar form at ion .

Dem yelin at ing opt ic n eu rit is is due to ph agocytosis of th e m yelin n er ve sh eath s an d su bsequ en t lay-dow n of fibrou s plaqu es. It affect s th e w h ite m at ter t racts, brain stem , an d spin al cord, bu t th e periph eral n er ves are spared .

Presentation

Presen tat ion is u su ally at age 20 to 30 years an d is subacute an d u n ilateral. Th ere is decreased vision , pain exacerbated by eye m ovem en ts, an d t iny flash es of ligh t (ph osph en es). Visu al acuit y usually varies bet w een 20/60 (6/18) to 20/200 (6/60) in ret robulbar n eurit is an d papillit is. Afferen t pu pillar y defect , dysch rom atopsia (n ot proport ion al to visu al loss), dim in ish ed ligh t an d brigh t n ess appreciat ion , an d decreased con t rast sen sit ivit y are presen t . Th ere is diffuse depression of th e cen - t ral 30 degrees of th e visu al field .

Dem yelin at ing diseases causing ocular involvem en t in clude th e follow ing:

Mult iple sclerosis: Most com m on . MRI sh ow s p eriven t ricu lar plaques.

Devic disease (neurom yelit ica opt ica): Bilateral opt ic n eurit is w ith t ran sverse m yelit is. Severe bilateral dim in ish ed visual acuit y w ith paraplegia.

Schilder disease: On set before 10 years an d death w ith in 1 to 2 years. Bilateral, progressive opt ic n eurit is.

Differential Diagnosis

Com pressive opt ic lesion s, sarcoidosis, vascu lit is (e.g., system ic lu pus er yth em atosu s), syph ilis, an terior isch em ic opt ic n europathy (AION), Leber’s h ereditar y opt ic n europathy (LHON), toxic or n ut rit ion al n eu ropath ies, post viral dem yelin at ion

Management

Presentation

Presen tat ion is u sually at age 20 to 30 years an d is su bacu te an d u n ilateral. Diag- n osis is usu ally clin ical. Th ere is decreased vision , p ain exacerbated by eye m ove- m en ts, an d t iny flash es. In th e case of an at ypical presen t at ion , progressive opt ic n europ athy n eeds to be ru led ou t .

Invest igat ion s requ ired in clude MRI, ch est x-ray, serological tests an t in u clear an t ibody (ANA), er yth rocyte sedim en t at ion rate, syph ilis, an t in eut roph il cytoplas- m ic an t ibodies (ANCA), an d cerebrospin al fluid (CSF) an alysis. Typ ical w h ite m atter plaques in MRI w ith oligoclon al ban ds in CSF suggest a dem yelin at ing cau se. Treat m en t m odalit ies in clude in t raven ou s m ethylpredn isolon e 1 g daily for 3 days follow ed by oral predn isolon e 1 m g/kg/day for 11 days, th en t apered over 3 days. Th e resu lts of th e ONTT (opt ic n eu rit is t reat m en t t rial) sh ow th at th e above regi- m en h asten s visual recover y, but does n ot affect long term visu al outcom es. In t ra- m u scular in terferon β-1a at th e first episode delays th e developm en t of m u lt iple sclerosis.

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Differential Diagnosis

In t racran ial space-occu pying lesion s (t um ors, h em orrh age, abscess, etc.), in filt ra- t ive (leukem ia, lym ph om a), in flam m ator y (opt ic n eurit is, sclerit is, etc.), gran ulo- m atou s (sarcoidosis, t u bercu losis, etc.), an d vascu lar causes (AION, diabet ic papillopathy, etc.) of disk sw elling an d pseudopapilledem a

Management

Fundus fluorescein angiography (FFA) show s leakage from dilated disk capillaries, and late pooling of the dye is evident . Magnetic resonance venography is done to check the cerebral sinuses and lum bar puncture for CSF analysis. Treatm ent is directed tow ard the underlying cause; neurosurger y m ay be required. Regular ophthalm ic follow -up w ith disk status, vision, color vision, and field changes is required.

Pseudopapilledema

An om alies resem ble papilledem a but are n ot du e to raised ICT. Th ey can in stead be du e to disk drusen , t ilted disks, hyperopic disks, m yopic disks, an d m yelin ated peripapillar y n er ve fibers. Disk drusen is autosom al dom in an t , bilateral lum py disk w ith absen t cu p an d cen t rally em erging vessels w ith an abn orm al bran ch ing pat tern . Visu al acuit y is u su ally n orm al bu t associated field defects are usually presen t . Diagn osis is by autoflu orescen ce, B-scan , ult rason ography, an d CT scan .

Sim ilarly m yopic disks m ay be elevated n asally an d m ay leak on FFA. Hyperopic disk is crow ded an d elevated an d th us can lead to con fu sion . Tilted disks are elevated su perotem porally (Fig. 13.12).

Pseudotumor Cerebri

Pseu dot u m or cerebri is usually a ben ign , self-lim it ing disease of un kn ow n et iology ch aracterized by sign s an d sym ptom s of raised in t racran ial pressu re w ith n orm al CSF, pap illedem a, an d n orm al n er vous system im aging. It can be idiopath ic an d is kn ow n to be associated w ith vit am in A in toxicat ion , tet racyclin e th erapy, steroid w ith draw al, n alidixic acid, im pairm en t of cen t ral ven ous drain age, an d system ic lupu s er yth em atosu s.

Fig . 13.12 Optic nerve head dru-

sen. (Courtesy of Robin D. Hamilton, AM Hamilton)

13 Neuro-Ophthamology 385

Presentation

Presen t ing an d diagn ost ic feat ures of pseu do t u m or cerebri are h olocran ial h ead - ach e, m on ocu lar or bin ocu lar blackout s of vision (du e to m icrocirculat ion s dis- t u rban ce at th e tem poral lobe), sixth -n er ve palsy w ith diplopia, t in n it u s, papilledem a, arcu ate scotom as in th e n asal region , an d relat ive afferen t pu pillar y defect (RAPD).

Differential Diagnosis

Brain t um ors (m ost com m on ly gliom a), ar terioven ous m alform at ion s, in fect iou s diseases su ch as viral en ceph alit is, an d an om alous opt ic n er ve w ith h eadach e

Management

Asym ptom at ic p at ien ts sh ould be follow ed up regu larly. Carbon ic an hydrase in - h ibitors (in h ibit th e CSF produ ct ion ), cor t icosteroids, oral hypoglycem ic agen ts, lum boperiton eal sh u n t , an d opt ic n er ve sh eath decom pression (ONSD) are oth er t reat m en t m odalit ies.

Arteritic Ischemic Optic Neuropathy (Giant Cell Arteritis)

Th is in farct ion of th e opt ic disk is associated w ith gian t cell arterit is (GCA). Tw en t y percen t of cases do n ot h ave any system ic sign s of GCA at th e t im e of presen t at ion (occult GCA).

Presentation

Th e pat ien t presen ts w ith sudden , profou n d, u n ilateral visual loss w ith periocu - lar pain (preceded by t ran sien t visu al loss an d flash es of ligh t u n like n on arterit ic isch em ic opt ic n europathy. Alt it u din al field defect is com m on in th ese pat ien ts. Pat ien ts m ay experien ce associated sym ptom s su ch as h eadach e, n eck pain , jaw pain w ith claudicat ion , scalp ten dern ess, fevers, w eigh t loss, an d m yalgias.

Th e age of th e pat ien t is m ore th an 55 years. Palp able an d ten der tem poral ar ter y, elevated er yth rocyte sedim en t at ion rate, an d coexist ing an em ia are also presen t .

Differential Diagnosis

Non ar terit ic isch em ic opt ic n eu ropathy, opt ic n eu rit is, opt ic n er ve com pression , opt ic n europathy, cen t ral ret in al vein occlu sion (CRVO).

Management

ESR, C-react ive protein (CRP), an d tot al blood cou n t-raised platelet s are th e blood invest igat ion s requ ired to diagn ose an d follow up w ith th e pat ien t . Tem p oral arter y biopsy sh ow s gran ulom atou s in flam m at ion w ith a grossly n arrow lu m en . FFA sh ow s severe hypoperfu sion of ch oroid .

Pat ien ts are t reated w ith in t raven ous m ethylpredn isolon e 1 g/day w ith 80 m g oral predn isolon e for 3 days. After 3 days, 60-m g an d 50-m g oral dose each for 1 w eek follow ed by gradu al redu ct ion of 5 m g each w eek t ill 10 m g/day, w h ich is th e m ain ten an ce dose. Azath ioprin e h as been t ried in pat ien ts in toleran t of steroids. Progn osis is poor in spite of early st art of steroids, an d it w orsen s w ith t im e an d su bsequen t episodes.

386 Color Atlas of Ophthalm ology

A B

Fig . 13.13 (A) Nonarteritic ischemic optic neuropathy (diabetic), color picture.

(B) Nonarteritic ischemic optic neuropathy (diabetic), fundus fluorescein angiography.

Nonarteritic Ischemic Optic Neuropathy

Th is n europathy occurs becau se of occlusion of th e sh or t posterior ciliar y ar teries. It is m ost com m on in elderly pat ien ts, w ith st ruct ural crow ding of th e opt ic n er ve h ead du e to a sm all or absen t physiological cu p . System ic hyper ten sion , hyperch o- lesterolem ia, diabetes m ellit u s, hypercoagulat ion st ates, an d silden afil in take are som e of th e predisposit ion s for th e con dit ion .

Presentation

Pat ien ts h ave a sudden un ilateral pain less visu al loss, par t icularly after aw aken - ing, associated w ith color vision abn orm alit ies (propor t ion al to visu al loss) an d in ferior alt it u din al field defects. Late stages p resen t w ith opt ic at rophy follow ing ch ron ic vision loss.

Disk pallor w ith disk edem a (diffu se or sectoral) w ith peripapillar y splin ter h em orrh ages is th e t ypical presen t at ion (Fig. 13.13).

Differential Diagnosis

AION, opt ic n eu rit is, opt ic n er ve com pression , opt ic n europathy, an d CRVO

Management

Lipid profile, blood glu cose, com plete blood cou n t , an t in uclear an t ibody, flu orescein t repon em a an t ibody (FTA)/ven ereal disease research laborator y test , ESR, an d C-react ive protein (to rule ou t GCA) m ust be don e for all pat ien t s. FFA sh ow s early disk hyperflu orescen ce, w h ich in creases in in ten sit y in th e late st ages. In term itten t blocked fluorescen ce is seen ow ing to splin ter h em orrh ages. Treat m en t con - sists of m an aging th e u n derlying m edical con dit ion . Progn osis is favorable w ith m ain ten an ce of good visu al acu it y in m ost pat ien ts.

Toxic or Nutritional Optic Neuropathy

Th is con dit ion is also referred to as tobacco-alcoh ol am blyopia because it is prevalen t in h eavy drin kers an d ch ron ic pip e or cigar sm okers w h o n eglect th eir diet . Oth er et iologies in clude th iam in e an d vit am in B12 deficien cy, m eth an ol, eth am bu - tol, ch loram ph en icol, ison iazide, rifam picin , lead, an d digit alis.

13 Neuro-Ophthamology 387

Presentation

Presen t at ion in cludes in sidious, progressive, bilateral visual im pairm en t w ith dysch rom atopsia. Th e opt ic disk m ay be n orm al or tem poral pallor w ith splin ter h em - orrh ages arou n d th e disk. Cen t rocecal scotom a (bet ter appreciated by red target in stead of w h ite) is presen t .

Differential Diagnosis

Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy, Leber opt ic n europathy, an d bilateral com pressive opt ic n europathy

Management

Blood invest igat ion s m igh t reveal an associated pern icious an em ia an d vit am in B12 deficien cy. Weekly inject ion s of 1000 un its of hydroxycobalam in for 10 w eeks along w ith m ult ivitam in s are th e m ain stay of th e t reat m en t . Abstain from drin k- ing an d sm oking. Progn osis is good w ith t reat m en t in th e early st ages.

Leber Optic Neuropathy

Leber opt ic n eu ropathy is a m atern ally t ran sm it ted t rait arising from p oin t m ut a- t ion s (m ost com m on ly 11778 m ut at ion ) in m itoch on drial DNA. It t ypically affect s m en (3:1) in th eir t w en t ies or th irt ies. It is u n ilateral in it ially w ith subsequ en t involvem en t of th e oth er eye.

Presentation

Pat ien ts presen t w ith pain less un ilateral gradual loss of vision . Th e oth er eye is involved su bsequen tly. Dysch rom atopsia is presen t . Th ere is a m ildly edem atou s, hyperem ic disk w ith telangiect at ic vessels in th e peripapillar y ret in a. Th e late stage presen t s w ith opt ic at rophy. Den se cen t ral or paracen t ral scotom a is seen . Th e con dit ion m ay be associated w ith sp ast icit y an d gait dist urban ces ow ing to th e in abilit y to detoxify cyan ide.

Differential Diagnosis

Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy, n ut rit ion al opt ic n europ athy, an d bilateral com pressive opt ic n europathy

Management

A detailed h istor y h elps in ru ling ou t oth er causes of opt ic n europ athy. CT scan an d MRI scan h elp to rule ou t a com pressive lesion . Con sider a blood test to detect m itoch on drial ch rom osom al m u t at ion s. Gen et ic coun seling h elps.

Compressive Optic Neuropathy

Com pressive opt ic n eu ropathy result s from com pression of th e pregen iculate por- t ion of th e opt ic n er ve. Com m on causes in clude Graves thyroid oph th alm opathy, m en ingiom as, orbit al space-occu pying lesion , pseu dot u m or, cran ioph ar yngiom a, an d pit uitar y t u m ors.

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Presentation

Visual acuit y, color vision , an d con t rast sen sit ivit y are u sually affected . In it ially disk sw elling is presen t , follow ed by at rophy in th e late st ages. Afferen t pu pillar y defect an d cen t ral an d arcu ate scotom as are com m on fin dings. Proptosis m ay or m ay n ot be associated .

Differential Diagnosis

Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy, n ut rit ion al opt ic n europ athy, an d Leber opt ic n eu ropathy

Management

CT scan an d MRI of th e orbit an d brain are perform ed to n ote th e com pressive lesion an d it s exten t . Treat m en t is directed tow ard th e u n derlying cau se of com - pression .

Infiltrative Optic Neuropathy

In filt rat ion of th e opt ic n er ve can be by n eoplast ic or in flam m ator y cells from lym p h om as, leu kem ias, sarcoidosis, syph ilis, t u berculosis (TB), fu ngal in fect ion , or m etast asis from lu ng or breast carcin om a.

Presentation

Pat ien ts presen t w ith progressive, bilateral, severe vision loss. In it ially th e disk is n orm al oph th alm oscopically but later m ay becom e sw ollen due to in filt rat ion at th e opt ic n er ve h ead .

Differential Diagnosis

Bilateral AION, hypoten sive sh ock, radiat ion injur y, com pressive opt ic n europathy, n ut rit ion al opt ic n europ athy, an d Leber opt ic n eu ropathy

Management

MRI of th e orbit an d brain , CSF an alysis, an d screen ing test s for gran u lom atou s disorders an d blood -related disorders are required to con firm th e cau se. Palliat ive radioth erapy for in filt rat ive n eoplasm s an d cor t icosteroids or an t im icrobials for in filt rat ive or in fect iou s disorders are advocated form s of t reat m en t .

Radiation Optic Neuropathy

Th e n eu ropathy is usu ally delayed by 2 years after st an dard doses of radiat ion but m ay be seen m any years after th e t reat m en t . Th e opt ic n er ve can be affected by radiat ion to th e eye, orbit , sin us, n asoph ar yn x, or brain .

Presentation

Bilateral decreased visu al acu it y is th e presen t ing sym ptom .

Differential Diagnosis

Bilateral AION, hypoten sive sh ock, in filt rat ive opt ic n europathy, com pressive opt ic n europ athy, n ut rit ion al opt ic n europathy, an d Leber opt ic n europ athy

13 Neuro-Ophthamology 389

Management

Th e h istor y is sign ifican t . CT an d MRI are don e to evalu ate th e opt ic n er ve. No t reat m en t is available.

Tumors of Neural Origin

Optic Nerve Glioma

Low -grade, pilocyt ic ast rocytom a can involve opt ic n er ve an d ch iasm ; 30%are associated w ith n eurofibrom a t yp e 1 (NF-1) an d h ave a bet ter progn osis.

Presentation

Opt ic n er ve gliom a u su ally presen ts in th e first decade w ith gradu al visu al loss associated w ith proptosis (<3 m m , axial, n onpulsat ile, n on redu cible). St rabism u s an d nyst agm u s can also be th e presen t ing com plain t s. Oth er feat u res in clude relat ive afferen t papillar y defect , opt ic at rophy, opt ic disk sw elling, optociliar y sh un ts, an d CRVO. In ch iasm al lesion s, hypoth alam ic an d pit uitar y dysfun ct ion sym ptom s an d sym ptom s of in creased in t racran ial pressu re m ay be presen t . Opt ic n er ve gliom a in adults is rare, bu t if presen t it ten ds to be aggressive an d fat al.

CT scan sh ow s a fusiform en largem en t of th e n er ve, kin king or irregular n er ve, an d region s of low in ten sit y w ith in th e n er ve. Th ere is n o calcificat ion or hyperostosis. MRI reveals hypoin ten se areas on T1-w eigh ted im ages an d hyperin ten se areas on T2-w eigh ted im ages. It m ay sh ow in t racran ial exten sion .

Histology sh ow s spin dle-sh aped ast rocytes w ith deeply eosin oph ilic large cell processes kn ow n as Rosen th al fibers w ith associated degen erat ive foci an d reac- t ive hyperplasia of m en inges.

Differential Diagnosis

Oth er n eoplasm s (m en ingiom a, lym ph angiom a, h em angiom a, rh abdom yosarcom a), acute eth m oidit is, hyp erthyroidism , cran iosten osis, an d t rau m a

Management

Th e t reat m en t lin e con sist s of obser vat ion for sm all t u m ors w ith good vision . Su r- gical excision w ith globe preser vat ion is recom m en ded for large t um ors con fin ed to th e orbit . Radioch em oth erapy is used for in t racran ial exten sion .

Neurofibroma and Schw annoma

NF is a ben ign t u m or d er ived from glial cells of p er ip h eral n er ves. En d on eu ral cells an d a xon s m ay also be involved in NF. Sch w an n om as ten d to be sin gu lar an d en cap su lated , w h ereas NFs are m ore likely to be m u lt ip le an d en cap su lated an d are m ore likely to u n d ergo m align an t t ran sfor m at ion . Plexifor m NFs (an ot h er t yp e) are alm ost alw ays associated w it h au tosom al d om in an t n eu rofibrom atosis t yp e 1 (NF-1, von Recklin gh au sen NF) lin ked to ch rom osom e 17, w h ich u su ally p resen t s in early ch ild h ood .

390 Color Atlas of Ophthalm ology

Presentation

Pat ien ts can presen t w ith a su bcut an eou s lu m p aroun d th e eye or w ith dou ble vision , decreased vision , an d proptosis in cases w ith in t raorbit al t um or. Mult ip le n eurofibrom as (NF-1) are associated w ith pigm en ted skin lesion s, osseou s lesion s, café-au -lait spot s, S-sh aped deform it y of th e lid, Lisch n odu les in th e iris, an d hypert rop hy of periocu lar t issu e, w h ich on palpat ion h as a “bag of w orm s” feeling.

Differential Diagnosis

Oth er n eoplasm s (opt ic n er ve gliom a, lym ph angiom a, h em angiom a, rh abdom yosarcom a), acu te eth m oidit is, hyper thyroidism , cran iosten osis, an d t raum a

Management

Oph th alm ic w orkup in clu des a com p lete m edical h istor y, assessm en t of visual acuit y, ocu lar m ovem en t s, displacem en t , exoph th alm om et r y, ton om et r y, dilated fun du s exam in at ion , CT scan , MRI, an d u lt rason ography. Sym ptom at ic t u m ors n eed excision .

Orbit al surger y sh ou ld n ot be don e becau se th e t u m or is related to im por tan t orbit al st r uct u res.

Meningioma

Middle-aged fem ales are affected . Presen tat ion s var y w ith th e site of th e lesion .

Presentation

Opt ic n er ve sh eath m en ingiom as are ben ign p roliferat ion s of m en ingoepith elial cells presen t ing w ith progressive m on ocular vision loss, opt ic at rophy, an d optociliar y sh un t s. Tu bercu lum sellae m en ingiom a com presses th e ju n ct ion of th e opt ic ch iasm w ith th e opt ic n er ve cau sing ipsilateral cen t ral scotom as w ith con t ralateral jun ct ion al scotom a. Sph en oidal ridge t um ors w ill com press th e opt ic n er ve, an d sim ilarly olfactor y lesion s w ill cau se com pression of th e opt ic n er ve an d loss of th e sen se of sm ell.

CT sh ow s diffu se t ubular en largem en t of th e opt ic n er ve seat , t ram t rack or railroad sign , an d calcificat ion . MRI reveals isoin ten se or hyperin ten se lesion on T1 an d T2. CT or MRI w ith con t rast is m ore h elpful.

Differential Diagnosis

Oth er in t raocular t u m ors, m et astasis, m u cocele, an d lym ph om a

Management

Pat ien ts w ith progressive field loss requ ire t reat m en t by a n eu rosu rgeon . Treat- m en t requires su rger y follow ed by radioth erapy.

Blepharospasm

Bleph arospasm is a t ype of facial dyston ia, m ain ly affect ing fem ales (2:1) an d associated w ith ton ic spasm s of orbicu laris ocu li (Fig. 13.14).